Hysterosalpingography in The Assessment of Congenital Cervical Anomalies

Cervical abnormalities may be congenital or acquired. Congenital anomalies of the cervix are rarely isolated, and more commonly accompany other uterine anomalies. Various imaging tools have been used in the assessment of Müllerian duct anomalies (MDAs). Currently, magnetic resonance imaging (MRI) is the modality of choice for definitive diagnosis and classification of these MDAs. Hysterosalpingography is a basic tool for evaluation of infertility and allows us to detect a spectrum of anatomical malformations of the utero-cervix in the setting of MDAs. It provides good outlines of the uterine cavity and fallopian tubes, as well as the cervical canal and isthmus. However, hysterosalpingograms (HSG) cannot be performed in patients with isolated congenital maldevelopment (agenesis/disgenesis) of the cervix. This part of pictorial review illustrates the various radiographic appearances of congenital malformations of the utero-cervix with a brief overview of the embryologic features. Accurate diagnosis of such cases is considered essential for optimal treatment and categorization of each anomaly.


Introduction
Female genital malformations include various forms of developmental and morphological malformations of the vagina, cervix, uterus, adnexa and associated malformations (VCUAM) (1). Genital malformations have an incidence of up to ~7% in the general population (2).
Congenital anomalies of the cervix are rarely isolated, and are more commonly associated with other uterine and vaginal anomalies. Cervical anomalies may manifest as agenesis, dysgenesis, obstruction, abnormal length, inadequate width, and hypertrophy (3); however, complete or partial duplication of the cervix with a normal uterus and an unusual Müllerian anomaly have been reported (4,5). Cervical atresia usually presents with primary amenorrhea and cyclic abdominal pain. Depending on the type and degree of maldevelopment of the uterine cervix, a woman's reproductive potential may be adversely affected.
Several imaging tools have been used in the assessment of Müllerian duct anomalies (MDAs). Although magnetic resonance imaging (MRI) is confor complex cases, hysterosalpingography is still an important tool in the early evaluation of infertility. Contrast medium which is slowly injected into the uterus through the cervical canal, provides good outlines of the uterine cavity and fallopian tubes, as well as the cervical canal and isthmus (6).
We retrospectively reviewed 38574 hysterosalpingograms (HSGs) performed over a 29-year pe-(G.Sh.). The indications for HSG included infertility, abnormal uterine bleeding, and symptoms the various radiographic appearances of congenital malformations of the utero-cervix with a brief overview of the embryologic features.
The female genital tract develops from a pair of Müllerian ducts between 6 and 12 weeks of gestation. The process involves three main stages: i. Development of both Müllerian ducts that form the fallopian tubes, uterus, and cervix and upper two thirds of the vagina, whereas failure of this stage results in agenesis/hypoplasia or a unicornuate uterus, ii. Fusion of the lower Müllerian ducts leads to formation of the uterus and cervix while defects in this phase result in a bicornuate uterus, and iii. Canalization and septal resorption of the central septum which results in a single uterine cavity and cervix, whereas failure of this stage leads to a septate or arcuate uterus. Mesonephric equate development, fusion, and resorption of the walls of Müllerian ducts (7). There is controversy over formation of the vagina. Recent studies have stated that the mesonephric ducts together with the Müllerian tubercle form the vagina (7, 8).
pleted. Development of both Müllerian ducts and the urinary tract occurs from a common ridge of the mesoderm; hence, anomalies of the urinary tract are commonly observed in females with genital malformation. malformation is necessary to prevent inadequate surgery and achieve optimal treatment. Until now, tract anomalies have been proposed: the American American Society for Reproductive Medicine, AFS/ASRM) (9); the embryological-clinical clascervix, uterus, adnexae and associated Malformations system based on the tumor node metastasis (TNM) principle in oncology (1), and the new European Society of Human Reproduction and Embryology (ESHRE) and European Society for seem to be associated with limitations, especially in the diagnosis of unusual and complex malformations (12). The accurate diagnosis of such cases is considered essential for optimal treatment and in support of the embryologic concept.
Agenesis/dysgenesis of the cervix is rare and usually occurs in association with complete or parcervical agenesis. Clinical examination has limited diagnostic value and in most cases hysterosalpingography is impossible.
Uterus didelphys results from complete fail-ure of Müllerian duct fusion and accounts for approximately 5% of MDAs (2). On hysterosalpingography, two symmetric separate cavities and two cervical canals are present; a double vagina is often present (Fig.1). Didelphys uterus is usually asymptomatic, while cases with unilateral vaginal obstruction may manifest with hematometrocolpos and dysmenorrhea at menarche. The bicornuate uterus represents approximately 25% of MDAs and results from incomplete fusion of the Müllerian ducts at the level of the uterine fundus (2). The two separate uterine cavities are fused caudally and communicate in the lower segment, mostly at the uterine isthmus; a single cervix and vagina are present. Hysterosalpingography demonstrates separate fusiform uterine horns, often with are various degrees of separation between the two horns, as follows: a complete bicornuate uterus, in which the failure to fuse extends the length of the uterine body inferiorly to the internal os; and lesser degrees of a bicornuate uterus, in which the partial interfering cleft is variable in length, extending from the fundus to the cervix (Fig.2).

B. C.
A septate uterus results from partial or complete failure of resorption of the uterovaginal septum after fusion of the paramesonephric ducts, which occurs in approximately 35% of MDAs (2). Hysterosalpingography of a septate uterus represents varying degrees of the midline septum, extending from the fundus to the cervix and upper vagina, and yielding a V-shaped the two uterine horns (Fig.3). In 25% of cases, complete extension of the septum to the upper vagina is present (Fig.3C) (14).

C.
Exposure to DES in utero results in multiple, benign abnormalities of the genital tract and clear cell adenocarcinoma of the vagina. DES has been associated with T-shaped and irregular configurations of the endometrial cavity, constrictive bands, structural cervical changes, and cervical anomalies that include hypoplasia, cervical ridges, and cervical collars (15).
Hysterosalpingography is an excellent screening tool to diagnose DES-related uterine anomalies. The radiographic appearance includes an irregular, narrowed endocervical canal with a shortened upper uterine segment and small, typically irregular cavity scheme for nine subtypes of septate and bicornuate between two separate uterocervical cavities. All types of communicating uteri have an isthmic communication, except for type 9, which has a low cervical communication.

Fig.4: -
During >29 years of one author's experience (G.SH) in performing HSGs, some rare cases of communicating uteri have been observed and reported (Fig.5). Of these, three represented communications at unusual a mid-corporeal communication, in which the septum ended inferiorly several millimeters above the external os (Fig.6). Another case was a bicornuate uterus with two sites of communication, at the mid-cervical and isthmic levels (Fig.7), and a third case with communication that involved the bicornuate uterus with a low cervical communication, left cervico-vaginal atresia, and left renal agenesis (Fig.8). uteri that communicate with double cervices with or without vaginal septa have been previously described (4,(17)(18)(19). We encountered a case of normal uterus with septate cervix and vagina (Fig.9).

Fig.9: -
In the second case, the proximal part of the cervical canal was double, but the distal portion was sin- The third case was a bicornuate uterus with communication at the isthmic level, a septate cervix, and normal external cervical os and vagina (Fig.11). The patient had no history of any previous vaginal or cervical septum resection.

Conclusion
Although congenital anomalies of the uterocervix in the setting of MDAs are rare, the impact on a woman's reproductive potential can be widely diagnosed by HSG. The diagnostic value of HSG in the detection of anomalies varies, depending on the type of malformation. Accurate diagnosis of these cases, especially the cases optimal treatment and categorization of each anomaly.